KEGG   PATHWAY: hsa04610Help
hsa04610                    Pathway                                

Complement and coagulation cascades - Homo sapiens (human)
The complement system is a proteolytic cascade in blood plasma and a mediator of innate immunity, a nonspecific defense mechanism against pathogens. There are three pathways of complement activation: the classical pathway, the lectin pathway, and the alternative pathway. All of these pathways generate a crucial enzymatic activity that, in turn, generates the effector molecules of complement. The main consequences of complement activation are the opsonization of pathogens, the recruitment of inflammatory and immunocompetent cells, and the direct killing of pathogens. Blood coagulation is another series of proenzyme-to-serine protease conversions, culminating the formation of thrombin, the enzyme responsible for the conversion of soluble fibrinogen to the insoluble fibrin clot. Protease-activated receptors, such as those activated by thrombin, are members of G protein-coupled receptors and function as a mediator of innate immunity. The kallikrein-kinin system is an endogenous metabolic cascade, triggering of which results in the release of vasoactive kinins (bradykinin-related peptides). Kinin peptides are implicated in many physiological and pathological processes including the regulation of blood pressure and sodium homeostasis, inflammatory processes, and the cardioprotective effects of preconditioning.
Organismal Systems; Immune system
BRITE hierarchy
Pathway map
hsa04610  Complement and coagulation cascades

Ortholog table
N00463  Alternative pathway of complement activation
H00102  Classic complement pathway component defects
H00103  Late complement pathway defects
H00104  Alternative complement pathway component defects
H00105  Mannose-binding lectin pathway component defects
H00106  Complement regulatory protein defects
H00219  Hemophilia
H00220  Factor V deficiency
H00222  Afibrinogenemia
H00223  Inherited thrombophilia
H00845  Familial amyloidosis
H00938  Factor XI deficiency
H00941  Factor XII deficiency
H00945  Factor XIII deficiency
H00983  Alpha-2-plasmin inhibitor (a2-PI) deficiency
H01006  Hereditary angioedema
H01078  Fletcher factor deficiency
H01103  Alpha-1-antitrypsin deficiency
H01106  Plasminogen activator inhibitor type 1 deficiency
H01206  Plasminogen deficiency
H01254  Congenital prothrombin deficiency
H01381  Antithrombin III deficiency
H01434  Atypical hemolytic uremic syndrome
H01726  Membranoproliferative glomerulonephritis
H01887  3MC syndrome
H02092  von Willebrand disease
H02108  Basal laminar drusen
H02240  Ehlers-Danlos syndrome periodontal type
H02256  Factor VII deficiency
H02257  Factor X deficiency
D00160  Aminocaproic acid (USP/INN)
D00181  Argatroban (USAN)
D00291  Desmopressin (INN)
D01136  Tranexamic acid (JP17/USAN/INN)
D01672  Gabexate mesilate (JP17)
D01844  Fondaparinux sodium (JAN/USAN/INN)
D01981  Ximelagatran (JAN/USAN/INN)
D02112  Heparin sodium (JP17/USP/INN)
D02235  Desmopressin acetate (USAN)
D02956  Anticoagulant heparin (USP)
D02980  Adomiparin sodium (USAN)
D03136  Bivalirudin (USAN/INN)
D03213  Apixaban (JAN/USAN/INN)
D03330  Kallidinogenase (JP17/INN)
D03337  Reviparin sodium (JAN)
D03353  Dalteparin sodium (JAN/USAN/INN)
D03644  Danaparoid sodium (JAN/USAN)
D03673  Deligoparin sodium (USAN/INN)
D03674  Enoxaparin sodium (JAN/USAN/INN)
D03677  Deltibant (USAN/INN)
D03692  Desirudin (USAN/INN)
D03722  Efegatran sulfate (USAN)
D03728  Napsagatran (USAN)
D03766  Diaplasinin (USAN)
D03931  Ecallantide (USAN/INN)
D03940  Eculizumab (USAN/INN)
D04029  Razaxaban hydrochloride (USAN)
D04183  Fidexaban (USAN/INN)
D04427  Heparin calcium (JP17)
D04492  Icatibant acetate (JAN/USAN)
D04977  Parnaparin sodium (JP17)
D06142  Tifacogin (USAN/INN)
D06398  Tinzaparin sodium (USAN)
D06410  Thrombomodulin alfa (INN)
D06880  Lepirudin (INN)
D07082  Dabigatran etexilate mesylate (USAN)
D07086  Rivaroxaban (JAN/USAN/INN)
D07143  Melagatran (INN)
D07144  Dabigatran etexilate (USAN/INN)
D07510  Adomiparin (USAN)
D07568  Aminomethylbenzoic acid
D08004  Gabexate (INN)
D08547  Sulodexide (INN)
D08656  Aleplasinin (USAN/INN)
D08873  Betrixaban (USAN)
D08913  Eribaxaban (USAN)
D09546  Edoxaban tosilate hydrate (JAN)
D09707  Dabigatran (USAN/INN)
D09710  Edoxaban (USAN/INN)
D09765  Vorapaxar (USAN/INN)
D09766  Vorapaxar sulfate (JAN/USAN)
D09817  Darexaban maleate (JAN)
D09866  Atopaxar (USAN)
D09867  Atopaxar hydrobromide (USAN)
D09923  Letaxaban (USAN/INN)
D10150  Pegnivacogin sodium (USAN)
D10418  Delparantag pentahydrochloride (USAN)
D10471  Idraparinux sodium (USAN)
D10484  Pegnivacogin (USAN)
D10821  Emicizumab (USAN/INN)
D10845  Conestat alfa (INN)
D11029  Andexanet alfa (USAN/INN)
D11054  Ravulizumab (USAN)
D11093  Avacopan (JAN/USAN/INN)
D11094  Lanadelumab (USAN)
D11160  Caplacizumab (USAN/INN)
D11261  Marstacimab (USAN)
D11280  Bivalirudin trifluoroacetate (USAN)
D11331  Olendalizumab (USAN)
Homo sapiens (human) [GN:hsa]
2152  F3; coagulation factor III, tissue factor [KO:K03901]
2155  F7; coagulation factor VII [KO:K01320] [EC:]
2159  F10; coagulation factor X [KO:K01314] [EC:]
2153  F5; coagulation factor V [KO:K03902]
2147  F2; coagulation factor II, thrombin [KO:K01313] [EC:]
2161  F12; coagulation factor XII [KO:K01328] [EC:]
2160  F11; coagulation factor XI [KO:K01323] [EC:]
2158  F9; coagulation factor IX [KO:K01321] [EC:]
7450  VWF; von Willebrand factor [KO:K03900]
2157  F8; coagulation factor VIII [KO:K03899]
7056  THBD; thrombomodulin [KO:K03907]
10544  PROCR; protein C receptor [KO:K06557]
5624  PROC; protein C, inactivator of coagulation factors Va and VIIIa [KO:K01344] [EC:]
2149  F2R; coagulation factor II thrombin receptor [KO:K03914]
2151  F2RL2; coagulation factor II thrombin receptor like 2 [KO:K04235]
9002  F2RL3; F2R like thrombin or trypsin receptor 3 [KO:K04236]
2162  F13A1; coagulation factor XIII A chain [KO:K03917] [EC:]
2165  F13B; coagulation factor XIII B chain [KO:K03906]
1361  CPB2; carboxypeptidase B2 [KO:K01300] [EC:]
2243  FGA; fibrinogen alpha chain [KO:K03903]
2244  FGB; fibrinogen beta chain [KO:K03904]
2266  FGG; fibrinogen gamma chain [KO:K03905]
3818  KLKB1; kallikrein B1 [KO:K01324] [EC:]
3827  KNG1; kininogen 1 [KO:K03898]
623  BDKRB1; bradykinin receptor B1 [KO:K03915]
624  BDKRB2; bradykinin receptor B2 [KO:K03916]
5340  PLG; plasminogen [KO:K01315] [EC:]
7035  TFPI; tissue factor pathway inhibitor [KO:K03909]
462  SERPINC1; serpin family C member 1 [KO:K03911]
3053  SERPIND1; serpin family D member 1 [KO:K03912]
5104  SERPINA5; serpin family A member 5 [KO:K03913]
5627  PROS1; protein S [KO:K03908]
5054  SERPINE1; serpin family E member 1 [KO:K03982]
5055  SERPINB2; serpin family B member 2 [KO:K19821]
5327  PLAT; plasminogen activator, tissue type [KO:K01343] [EC:]
5328  PLAU; plasminogen activator, urokinase [KO:K01348] [EC:]
5329  PLAUR; plasminogen activator, urokinase receptor [KO:K03985]
5265  SERPINA1; serpin family A member 1 [KO:K03984]
5345  SERPINF2; serpin family F member 2 [KO:K03983]
2  A2M; alpha-2-macroglobulin [KO:K03910]
629  CFB; complement factor B [KO:K01335] [EC:]
1675  CFD; complement factor D [KO:K01334] [EC:]
718  C3; complement C3 [KO:K03990]
727  C5; complement C5 [KO:K03994]
729  C6; complement C6 [KO:K03995]
730  C7; complement C7 [KO:K03996]
731  C8A; complement C8 alpha chain [KO:K03997]
732  C8B; complement C8 beta chain [KO:K03998]
733  C8G; complement C8 gamma chain [KO:K03999]
735  C9; complement C9 [KO:K04000]
712  C1QA; complement C1q A chain [KO:K03986]
713  C1QB; complement C1q B chain [KO:K03987]
714  C1QC; complement C1q C chain [KO:K03988]
715  C1R; complement C1r [KO:K01330] [EC:]
716  C1S; complement C1s [KO:K01331] [EC:]
4153  MBL2; mannose binding lectin 2 [KO:K03991]
5648  MASP1; mannan binding lectin serine peptidase 1 [KO:K03992] [EC:3.4.21.-]
10747  MASP2; mannan binding lectin serine peptidase 2 [KO:K03993] [EC:]
717  C2; complement C2 [KO:K01332] [EC:]
720  C4A; complement C4A (Rodgers blood group) [KO:K03989]
721  C4B; complement C4B (Chido blood group) [KO:K03989]
719  C3AR1; complement C3a receptor 1 [KO:K04009]
11326  VSIG4; V-set and immunoglobulin domain containing 4 [KO:K19822]
1378  CR1; complement C3b/C4b receptor 1 (Knops blood group) [KO:K04011]
1380  CR2; complement C3d receptor 2 [KO:K04012]
3684  ITGAM; integrin subunit alpha M [KO:K06461]
3689  ITGB2; integrin subunit beta 2 [KO:K06464]
3687  ITGAX; integrin subunit alpha X [KO:K06462]
728  C5AR1; complement C5a receptor 1 [KO:K04010]
3075  CFH; complement factor H [KO:K04004]
3426  CFI; complement factor I [KO:K01333] [EC:]
710  SERPING1; serpin family G member 1 [KO:K04001]
1604  CD55; CD55 molecule (Cromer blood group) [KO:K04006]
4179  CD46; CD46 molecule [KO:K04007]
722  C4BPA; complement component 4 binding protein alpha [KO:K04002]
725  C4BPB; complement component 4 binding protein beta [KO:K04003]
966  CD59; CD59 molecule (CD59 blood group) [KO:K04008]
1191  CLU; clusterin [KO:K17252]
7448  VTN; vitronectin [KO:K06251]
C00290  Fibrin
C00306  Bradykinin
Bajic G, Degn SE, Thiel S, Andersen GR
Complement activation, regulation, and molecular basis for complement-related diseases.
EMBO J 34:2735-57 (2015)
Mathern DR, Heeger PS
Molecules Great and Small: The Complement System.
Clin J Am Soc Nephrol 10:1636-50 (2015)
Merle NS, Church SE, Fremeaux-Bacchi V, Roumenina LT
Complement System Part I - Molecular Mechanisms of Activation and Regulation.
Front Immunol 6:262 (2015)
Oikonomopoulou K, Ricklin D, Ward PA, Lambris JD
Interactions between coagulation and complement--their role in inflammation.
Semin Immunopathol 34:151-65 (2012)
Kurosawa S, Stearns-Kurosawa DJ
Complement, thrombotic microangiopathy and disseminated intravascular coagulation.
J Intensive Care 2:65 (2014)
Hillmeister P, Persson PB
The Kallikrein-Kinin system.
Acta Physiol (Oxf) 206:215-9 (2012)
Rau JC, Beaulieu LM, Huntington JA, Church FC
Serpins in thrombosis, hemostasis and fibrinolysis.
J Thromb Haemost 5 Suppl 1:102-15 (2007)
Crooks MG, Hart SP
Coagulation and anticoagulation in idiopathic pulmonary fibrosis.
Eur Respir Rev 24:392-9 (2015)
Christiaans SC, Wagener BM, Esmon CT, Pittet JF
Protein C and acute inflammation: a clinical and biological perspective.
Am J Physiol Lung Cell Mol Physiol 305:L455-66 (2013)
Alberelli MA, De Candia E
Functional role of protease activated receptors in vascular biology.
Vascul Pharmacol 62:72-81 (2014)
Smith HW, Marshall CJ
Regulation of cell signalling by uPAR.
Nat Rev Mol Cell Biol 11:23-36 (2010)
Ricklin D, Lambris JD
Complement in immune and inflammatory disorders: pathophysiological mechanisms.
J Immunol 190:3831-8 (2013)
Ricklin D, Lambris JD
Complement in immune and inflammatory disorders: therapeutic interventions.
J Immunol 190:3839-47 (2013)
Loof TG, Deicke C, Medina E
The role of coagulation/fibrinolysis during Streptococcus pyogenes infection.
Front Cell Infect Microbiol 4:128 (2014)
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